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This study aims to define the morphological profile associated with the presence of donor-specific antibodies (DSAs) and/or C4d immunostaining in ABO-identical or compatible pediatric liver grafts. Ten-year protocol liver graft biopsies performed at 131.3 ± 15.3 months after transplantation in 53 pediatric liver graft recipients were reviewed. Immunostaining for C4d was systematically performed and semiquantitatively analyzed. DSAs were concurrently quantified, and results were available for 44 patients. All biopsies demonstrated fibrotic changes with a mean liver allograft fibrosis score (LAFSc) of 5.1 ± 2.2. A total of 31 (58%) biopsies exhibited C4d positivity. DSAs were detected in 20 (45%) patients, and mean maximal mean fluorescence intensity was 12,977 ± 6731. LAFSc (6.3 ± 1.3 versus 3.9 ± 2.2; P = 0.008), perivenular fibrosis (2.7 ± 0.5 versus 1.3 ± 1.0; P < 0.001), and portal inflammation (1.4 ± 0.8 versus 0.3 ± 0.5; P = 0.009) were significantly higher in the double-DSA and C4d-positive group versus the double-negative group. We defined a histological scoring system from these results, which was integrated with the 2016 Banff definition and allowed reclassifying patients for the diagnosis of chronic active antibody-mediated rejection (cAMR; 11/53 versus 13/53). Diagnoses of probable cAMR according to Banff 2016 (n = 4) were unchanged, but 2 among the 9 patients classified as possible cAMR according to the 2016 Banff definition were excluded for this diagnostic when using our histological score. In conclusion, our results confirmed that perivenular fibrosis and portal inflammation in late pediatric liver graft biopsies are features of cAMR. Our histological score could improve the accuracy of the 2016 Banff definition for the diagnosis of cAMR. Liver Transplantation 24 897-907 2018 AASLD., (© 2018 by the American Association for the Study of Liver Diseases.)

Background: Esophageal varices-related GI bleeding occurs frequently and early in life in children with biliary atresia and it may be life threatening., Objective: We report the results of prophylactic sclerotherapy in 13 infants with biliary atresia and large varices., Patients: Mean age was 13 months, mean weight was 8.2 kg, mean total serum bilirubin was 258 mumol/L, and mean prothrombin time was 78%. Esophageal varices were grade III (11 patients) or II (2 patients), with red signs in all infants and gastric varices in 12. None had GI bleeding., Intervention: Sclerotherapy was performed with the patient under continuous intravenous octreotide therapy in 7 infants., Results: In 8 children a complete or almost complete eradication of varices was obtained; none of these children bled later, 4 underwent liver transplantation, 3 are alive without liver transplantation, and 1 died of sepsis after 9 months awaiting liver transplantation. In 4 children a partial eradication was obtained and liver transplantation was performed. None of these children bled. One other child bled to death after 2 sessions of sclerotherapy., Limitations: Four ulcers and 2 stenoses occurred in 6 children with no octreotide versus no ulcer and 1 stenosis in 7 children receiving octreotide., Conclusion: These results (1) indicate that primary prevention of GI bleeding by sclerotherapy of esophageal varices is technically feasible and fairly effective in infants with biliary atresia and large varices, even in those with end-stage liver disease, (2) suggest that decreasing the risk of bleeding may allow liver transplantation under better conditions, and (3) further suggest that octreotide associated with sclerotherapy lowers the rate of complications.

Background: Hereditary tyrosinemia type I is a disease with a severe prognosis. Main causes of death are acute liver failure, neurologic crises and hepatocarcinoma. NTBC, which acts as an inhibitor of the 4-hydroxyphenylpyruvate dioxygenase, prevents the formation of toxic metabolites involved in hepatic, renal and neurologic lesions., Case Reports: Results of NTBC therapy used in three infants with type I tyrosinemia who presented with acute liver failure are reported. The diagnosis relied on the finding of high plasmatic levels of tyrosine and methionine, and abnormal urinary excretion of succinyl acetone and delta aminolevulinic acid. Treatment with NTBC was initiated within 2 to 8 days from onset of symptoms. Signs of liver failure resolved after 3 weeks therapy. After 12 to 39 months of follow-up, outcome remains favorable., Conclusion: The results reported here highlight the efficiency of NTBC in type I tyrosinemia with early acute onset. However, the long term outcome needs to be determined with regards to prevention of hepatocarcinoma and toxicity of the drug.